Ewing sarcoma family of tumors
Updates to Article Attributes
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Ewing sarcoma family of tumours (ESFT)are a group of small round blue cell tumours that are closely histogenetically related, all demonstrating non-random t(11;22)(q24;q12) chromosome rearrangement resulting in the formation of the EWS-ETS fusion gene 1-3. They include:
Terminology
Although the literature is littered with contradictory terminology, all three tumours are likely to represent the same entity with minor differences in differentiation. For example, PNET is more differentiated demonstrating neural elements (e.g. Homer Wright rosettes) 2.
As As such all three entities should be referred to as Ewing sarcoma, with pPNET / Askin representing extraskeletal Ewing sarcoma.
Pathology
Ewing sarcoma family of tumours include:
-<p><strong>Ewing sarcoma family of tumours (ESFT)</strong> are a group of <a href="/articles/small-round-blue-cell-tumours">small round blue cell tumours</a> that are closely histogenetically related, all demonstrating non-random t(11;22)(q24;q12) chromosome rearrangement resulting in the formation of the EWS-ETS fusion gene <sup>1-3</sup>. They include:</p><ol>- +<p><strong>Ewing sarcoma family of tumours </strong>are a group of <a href="/articles/small-round-blue-cell-tumours">small round blue cell tumours</a> that are closely histogenetically related, all demonstrating non-random t(11;22)(q24;q12) chromosome rearrangement resulting in the formation of the EWS-ETS fusion gene <sup>1-3</sup>. </p><h4>Terminology</h4><p>Although the literature is littered with contradictory terminology, all three tumours are likely to represent the same entity with minor differences in differentiation. For example, PNET is more differentiated demonstrating neural elements (e.g. Homer Wright rosettes) <sup>2</sup>. As such all three entities should be referred to as <a href="/articles/ewing-sarcoma">Ewing sarcoma</a>, with pPNET / Askin representing <a href="/articles/extraskeletal-ewing-sarcoma">extraskeletal Ewing sarcoma</a>.</p><h4>Pathology</h4><p>Ewing sarcoma family of tumours include:</p><ol>
-<a href="/articles/extraskeletal-ewing-sarcoma">extraosseous Ewing sarcoma</a> (EES)</li>- +<a href="/articles/extraskeletal-ewing-sarcoma">extraosseous Ewing sarcoma</a> (EES)</li>
-</ol><p>Although the literature is littered with contradictory terminology, all three tumours are likely to represent the same entity with minor differences in differentiation. For example, PNET is more differentiated demonstrating neural elements (e.g. Homer Wright rosettes) <sup>2</sup>. </p><p>As such all three entities should be referred to as <a href="/articles/ewing-sarcoma">Ewing sarcoma</a>, with pPNET / Askin representing <a href="/articles/extraskeletal-ewing-sarcoma">extraskeletal Ewing sarcoma</a>.</p>- +</ol>
Tags changed:
- cases
Systems changed:
- Oncology
- Musculoskeletal
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